2023
PLCG2-associated immune dysregulation (PLAID) comprises broad and distinct clinical presentations related to functional classes of genetic variants
Baysac K, Sun G, Nakano H, Schmitz E, Cruz A, Fisher C, Bailey A, Group P, Abbott J, Becerra J, Allenspach E, Assing K, Atkinson T, Bargir U, Baxter S, Bergerson J, Bista R, Blanche S, Buckley L, Butte M, Carcamo B, Chandrakala S, Chen K, Chervinskiy S, Chinn I, Chong H, Coffey K, Copland A, Cowen E, Cros G, De Bruycker J, de la Morena M, Ehlayel M, Satter L, Gelfand E, Gilliaux O, Glover S, Gorman M, Griffin T, Grimbacher B, Gru A, Haddad E, Hadjadj J, Hajjar J, Hauck F, Hautala T, Holland S, Hsieh E, Hsu F, Jacquemin E, Jindal A, Kahn S, Keller M, Kobayashi R, Krupski C, Larkin A, Lawrence M, Madkaikar M, Malphettes M, Martelius, Mehta M, Metcalfe D, Meyts I, Nannapaneni N, Vinyals J, Olivier K, Ombrello A, Orange J, Rabinovitch N, Rauscher C, Redfern A, Reynolds P, Rieux-Laucat F, Secord E, Seeborg F, Seppänen M, Sereti I, Shin D, Shin J, Snapper S, Suri D, Tangcheewinsirikul S, Thatayatikom A, Torgerson T, Touzot F, Uzel G, Varjosalo M, Vasconcelos D, von Bernuth H, Walsh T, Walter J, Ward B, Wittkowski H, Wysocki C, Mace E, Milner J, Ombrello M. PLCG2-associated immune dysregulation (PLAID) comprises broad and distinct clinical presentations related to functional classes of genetic variants. Journal Of Allergy And Clinical Immunology 2023, 153: 230-242. PMID: 37769878, DOI: 10.1016/j.jaci.2023.08.036.Peer-Reviewed Original ResearchConceptsImmune dysregulationPhospholipase C gamma 2Gain of functionB cellsNatural killer cell dysfunctionCalcium fluxLoF variantsNatural killer cellsHumoral immune deficiencyDistinct clinical presentationsPrimary patient cellsExtracellular signal-regulated kinase (ERK) phosphorylationB cell activationSignal-regulated kinase phosphorylationPLCG2 mutationsAntibody deficiencyKiller cellsClinical presentationImmune deficiencyCell dysfunctionClinical dataStandardized questionnaireFunctional alterationsSporadic casesPathogenic variantsA safe, effective, and single-day protocol for rapid drug desensitization to intravenous iron dextran
Belmont A, Liao J, Hsu F, Kwah J. A safe, effective, and single-day protocol for rapid drug desensitization to intravenous iron dextran. The Journal Of Allergy And Clinical Immunology In Practice 2023, 11: 3242-3244.e1. PMID: 37329952, DOI: 10.1016/j.jaip.2023.06.018.Peer-Reviewed Original Research
2022
Considerations in the management of hereditary angioedema due to C1-INH deficiency in women of childbearing age
Hsu FI, Lumry W, Riedl M, Tachdjian R. Considerations in the management of hereditary angioedema due to C1-INH deficiency in women of childbearing age. Allergy, Asthma & Clinical Immunology 2022, 18: 64. PMID: 35831891, PMCID: PMC9281160, DOI: 10.1186/s13223-022-00689-9.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsHereditary angioedemaHealth statusAdverse fetal outcomesCohort of patientsChoice of therapyContinuity of careMedical Advisory BoardC1-INH deficiencyWomen's health statusRisk-benefit assessmentHAE attacksHAE managementFetal outcomesProphylactic therapyPatient populationTreatment optionsFemale sexDisease burdenTreatment decisionsContraceptive useUnpredictable episodesTreatment efficacyMucous membranesGynecologic specialistsPregnancyDefining Clinical and Immunological Predictors of Poor Immune Responses to COVID-19 mRNA Vaccines in Patients with Primary Antibody Deficiency
Shin JJ, Par-Young J, Unlu S, McNamara A, Park HJ, Shin MS, Gee RJ, Doyle H, Afinogenova Y, Zidan E, Kwah J, Russo A, Mamula M, Hsu FI, Catanzaro J, Racke M, Bucala R, Wilen C, Kang I. Defining Clinical and Immunological Predictors of Poor Immune Responses to COVID-19 mRNA Vaccines in Patients with Primary Antibody Deficiency. Journal Of Clinical Immunology 2022, 42: 1137-1150. PMID: 35713752, PMCID: PMC9203263, DOI: 10.1007/s10875-022-01296-4.Peer-Reviewed Original ResearchMeSH KeywordsAntibodies, ViralCD8-Positive T-LymphocytesCommon Variable ImmunodeficiencyCOVID-19COVID-19 VaccinesHumansImmunity, CellularImmunoglobulin AImmunoglobulin GMRNA VaccinesPrimary Immunodeficiency DiseasesRNA, MessengerSARS-CoV-2Spike Glycoprotein, CoronavirusVaccinationVaccinesVaccines, SyntheticConceptsCommon variable immune deficiencyT cellsImmune responseIgG responsesCVID patientsMRNA vaccinesB cellsCoronavirus disease 2019 (COVID-19) mRNA vaccinesCOVID-19 mRNA vaccinesBaseline immune profileHistory of autoimmunityPrimary antibody deficiencyT cell responsesCellular immune responsesPoor immune responseVariable immune deficiencyMemory B cellsSARS-CoV-2 spike proteinBaseline IgGCVID diagnosisEM CD8Immunological predictorsPAD cohortSpecific CD4Immune profileClinical profile and treatment outcomes in patients with hereditary angioedema with normal C1 esterase inhibitor
Jones DH, Bansal P, Bernstein JA, Fatteh S, Harper J, Hsu FI, O’Connor M, Park N, Suez D. Clinical profile and treatment outcomes in patients with hereditary angioedema with normal C1 esterase inhibitor. World Allergy Organization Journal 2022, 15: 100621. PMID: 35145604, PMCID: PMC8804245, DOI: 10.1016/j.waojou.2021.100621.Peer-Reviewed Original ResearchManagement of HAEMost patientsHereditary angioedemaC1 esterase inhibitorAngioedema attacksHAE attacksMedian ageClinical profileSerum levelsRhC1-INHTreatment outcomesRecombinant human C1 esterase inhibitorNormal C1 esterase inhibitorEsterase inhibitorHuman C1 esterase inhibitorAcute HAE attacksHigh-dose antihistaminesUS treatment centersPatient-reported assessmentsLow serum levelsPatient-reported outcomesC1-INH deficiencyAvailability of treatmentImproved disease controlBreakthrough attacks
2021
Low IgG trough and lymphocyte subset counts are associated with hospitalization for COVID-19 in patients with primary antibody deficiency
Kuster JK, Unlu S, Makin TA, Par-Young J, Simonov M, Shafi S, Balanda M, Randolph C, Steele R, Hsu FI, Price C, Kohli-Pamnani A, Borish L, Lawrence MG, Kang I, Shin JJ. Low IgG trough and lymphocyte subset counts are associated with hospitalization for COVID-19 in patients with primary antibody deficiency. The Journal Of Allergy And Clinical Immunology In Practice 2021, 10: 633-636.e3. PMID: 34929372, PMCID: PMC8683251, DOI: 10.1016/j.jaip.2021.11.030.Peer-Reviewed Original Research
2020
Clinical presentation of hereditary angioedema.
Azmy V, Brooks JP, Hsu FI. Clinical presentation of hereditary angioedema. Allergy & Asthma Proceedings 2020, 41: s18-s21. PMID: 33109320, DOI: 10.2500/aap.2020.41.200065.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsHereditary angioedemaHAE attacksSevere abdominal painHalf of patientsAutosomal dominant diseaseC1 inhibitor proteinLaryngeal angioedemaOropharyngeal swellingAbdominal attacksAbdominal painErythema marginatumSignificant hypotensionLaryngeal edemaPrompt diagnosisClinical presentationMinor traumaSignificant morbiditySubmucosal edemaBowel soundsPhysical examinationRecurrent episodesGenitourinary tractFrequent precipitantUnpredictable severityVariable presentationDevelopment of the Asthma Impairment and Risk Questionnaire (AIRQ): A Composite Control Measure
Murphy K, Chipps B, Beuther D, Wise R, McCann W, Gilbert I, Eudicone J, Gandhi H, Harding G, Coyne K, Zeiger R, Board P, Kobernick A, Mohammad A, Cherry A, Fein A, Gaines A, Ramsey A, Michaud A, Palmer A, Kim A, Smith A, White A, Ricci A, Kettelhut B, Modena B, Stone B, Rolston B, Schnapf B, Krajicek B, Kraft C, Hopkins C, Natalie C, Anderson C, Czajkowski C, Arce D, Hamlin D, Hill D, Mares D, Pham D, Liao D, Simmons D, Johnson D, Jhaveri D, Maselli D, Kerwin E, Schuman E, Sher E, Bleecker E, Rakowski E, Hsu F, Averill F, Barsanti F, Gross G, Chupp G, Head G, Nasir H, Lee H, Barjaktarevic I, Cook J, Krainson J, Pearl J, Bellak J, Perez-Fernandez J, Rehm J, Trevor J, Most J, Hanson J, Blessing-Moore J, Biller J, Gedell J, Southard J, Chancellor J, Ilowite J, Romeo J, Mercado J, Fisher J, Greiwe J, Gregory K, Nadeau K, Shenoy K, Tan L, Stefanowicz L, Humes L, Bowers L, O’Connor M, Perez M, White M, Hegewald M, Wilson M, Sikora M, Morganroth M, Smith M, Farooqui N, Lesch N, Jain N, Parikh N, Hartog N, Luthin P, Stewart P, O’Connor P, Schochet P, Goldberg P, Patel P, Chinthrajah R, Sidhu R, Ayinla R, Brown R, Stadtler R, Wachs R, Balkissoon R, Duplantier R, Puente R, Settipane R, Mustafa S, Surani S, Jawad S, Adams S, Villegas S, Al-Farra S, Subramanian S, Bains S, Kelsen S, Wise S, Estrella-Eades S, Smith S, Qureshi T, Chen T, Kelbel T, Miller T, Jain V, Soong W, Soler X, Mattar Z. Development of the Asthma Impairment and Risk Questionnaire (AIRQ): A Composite Control Measure. The Journal Of Allergy And Clinical Immunology In Practice 2020, 8: 2263-2274.e5. PMID: 32387166, DOI: 10.1016/j.jaip.2020.02.042.Peer-Reviewed Original ResearchConceptsAsthma controlAsthma Control Test scoresAsthma control toolPhysician-diagnosed asthmaCut pointsLogistic regression analysisAsthma impairmentExacerbation historyAsthma severityFuture exacerbationsTest-retest reliabilityRisk QuestionnaireSymptom impairmentAsthmaSpecialty practicePatientsRisk questionsGlobal InitiativeImpairmentAirQRisk increaseCharacteristic curveExacerbationRegression analysisComposite measureExperience with Intravenous Plasma-Derived C1-Inhibitor in Pregnant Women with Hereditary Angioedema: A Systematic Literature Review
Brooks JP, Radojicic C, Riedl MA, Newcomer SD, Banerji A, Hsu FI. Experience with Intravenous Plasma-Derived C1-Inhibitor in Pregnant Women with Hereditary Angioedema: A Systematic Literature Review. The Journal Of Allergy And Clinical Immunology In Practice 2020, 8: 1875-1880.e3. PMID: 32251736, DOI: 10.1016/j.jaip.2020.03.009.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsPlasma-derived C1-INHHereditary angioedemaC1-INHPregnant womenPlasma-derived C1 inhibitorC1 inhibitorTreatment guideline recommendationsFirst-line treatmentFavorable safety profileNumber of dosesEnglish-language articlesIntravenous plasmaGuideline recommendationsThird trimesterConsensus guidelinesSafety profileSpontaneous abortionPregnancySystematic reviewLanguage articlesAngioedemaLiterature searchData extractionTrimesterWomenImmunological and Clinical Phenotyping in Primary Antibody Deficiencies: a Growing Disease Spectrum
Shin JJ, Liauw D, Siddiqui S, Lee J, Chung EJ, Steele R, Hsu FI, Price C, Kang I. Immunological and Clinical Phenotyping in Primary Antibody Deficiencies: a Growing Disease Spectrum. Journal Of Clinical Immunology 2020, 40: 592-601. PMID: 32239366, PMCID: PMC7260109, DOI: 10.1007/s10875-020-00773-y.Peer-Reviewed Original ResearchConceptsCommon variable immunodeficiencyInterstitial lung diseasePrimary antibody deficiencyConnective tissue diseaseAntibody deficiencyLow NK cell countHematopoietic malignanciesNon-infectious complicationsNon-infectious manifestationsNK cell countsNatural killer cellsPneumococcal vaccine responsesElectronic medical recordsResultsAll patientsImmune alterationsVaccine responsesKiller cellsTissue diseaseImmunoglobulin levelsVariable immunodeficiencyLung diseasePAD groupIgG deficiencyImmunological profileMedical recordsLong-term efficacy and safety of subcutaneous C1-inhibitor in women with hereditary angioedema: subgroup analysis from an open-label extension of a phase 3 trial
Levy DS, Farkas H, Riedl MA, Hsu FI, Brooks JP, Cicardi M, Feuersenger H, Pragst I, Reshef A. Long-term efficacy and safety of subcutaneous C1-inhibitor in women with hereditary angioedema: subgroup analysis from an open-label extension of a phase 3 trial. Allergy, Asthma & Clinical Immunology 2020, 16: 8. PMID: 32042283, PMCID: PMC7001333, DOI: 10.1186/s13223-020-0409-3.Peer-Reviewed Original ResearchOpen-label extensionC1-INHHealthy babyFemale subjectsFirst trimesterSubgroup analysisHereditary angioedemaHAE-C1INHIU/Severe angioedema attacksSubcutaneous C1-INHPhase 3 trialParallel-arm trialAttacks/monthLong-term efficacyFemale sex hormonesPre-study periodSubgroup of subjectsHAE attacksRoutine prophylaxisEfficacy endpointEligible subjectsUneventful pregnancyAngioedema attacksSex hormones
2019
Recombinant human C1 esterase inhibitor as short-term prophylaxis in patients with hereditary angioedema
Valerieva A, Staevska M, Jesenak M, Hrubiskova K, Sobotkova M, Zachova R, Hakl R, Andrejevic S, Suiter T, Grivcheva-Panovska V, Karadza-Lapic L, Soteres D, Shapiro R, Rumbyrt J, Tachdjian R, Mehta V, Hsu FI, Zanichelli A. Recombinant human C1 esterase inhibitor as short-term prophylaxis in patients with hereditary angioedema. The Journal Of Allergy And Clinical Immunology In Practice 2019, 8: 799-802. PMID: 31437566, DOI: 10.1016/j.jaip.2019.08.011.Peer-Reviewed Original ResearchEtoposide phosphate for pediatric orthopedic malignancies after intravenous etoposide hypersensitivity
Brooks JP, Azmy V, Thompson A, Luon D, Prozora SD, Price C, Hsu FI. Etoposide phosphate for pediatric orthopedic malignancies after intravenous etoposide hypersensitivity. Journal Of Oncology Pharmacy Practice 2019, 26: 228-231. PMID: 30885040, DOI: 10.1177/1078155219836478.Peer-Reviewed Original ResearchConceptsOrthopedic malignanciesHypersensitivity reactionsEtoposide phosphatePediatric patientsEtoposide hypersensitivityLarge academic medical centerAcademic medical centerCase of etoposideContinued therapyChart reviewFurther therapyFirst dosesFull dosesEwing's sarcomaMedical CenterPatientsMalignancySafe alternativeEtoposideHypersensitivityTherapyDiphenhydramineDosesPremedicationSarcoma
2018
Patients with common variable immunodeficiency with autoimmune cytopenias exhibit hyperplastic yet inefficient germinal center responses
Romberg N, Le Coz C, Glauzy S, Schickel JN, Trofa M, Nolan BE, Paessler M, Xu M, Lambert MP, Lakhani SA, Khokha MK, Jyonouchi S, Heimall J, Takach P, Maglione PJ, Catanzaro J, Hsu FI, Sullivan KE, Cunningham-Rundles C, Meffre E. Patients with common variable immunodeficiency with autoimmune cytopenias exhibit hyperplastic yet inefficient germinal center responses. Journal Of Allergy And Clinical Immunology 2018, 143: 258-265. PMID: 29935219, PMCID: PMC6400323, DOI: 10.1016/j.jaci.2018.06.012.Peer-Reviewed Original ResearchConceptsCommon variable immunodeficiencyVariable immunodeficiencyB cellsCommensal bacteriaIsotype-switched memory B cellsRegulatory T cell frequencyFollicular helper T cellsGC responseIsotype-switched antibodiesT cell frequenciesSubset of patientsT cell compartmentHelper T cellsPeripheral blood samplesMemory B cellsGerminal center responseB cell clonesAutoimmune cytopeniasGC hyperplasiaSerum endotoxemiaExcisional lymphAntibody responseT cellsMucosal microbiotaSomatic hypermutation frequencies
2016
Safety, Costs, and Efficacy of Rapid Drug Desensitizations to Chemotherapy and Monoclonal Antibodies
Sloane D, Govindarajulu U, Harrow-Mortelliti J, Barry W, Hsu FI, Hong D, Laidlaw T, Palis R, Legere H, Bunyavanich S, Breslow R, Wesemann D, Barrett N, Brennan P, Chong HJ, Liu A, Fernandez J, Fanning L, Kyin T, Cahill K, Bankova L, Lynch A, Berlin S, Campos S, Fuchs C, Mayer R, Matulonis U, Castells M. Safety, Costs, and Efficacy of Rapid Drug Desensitizations to Chemotherapy and Monoclonal Antibodies. The Journal Of Allergy And Clinical Immunology In Practice 2016, 4: 497-504. PMID: 26895621, DOI: 10.1016/j.jaip.2015.12.019.Peer-Reviewed Original ResearchConceptsRapid drug desensitizationDrug desensitizationAllergic patientsLife expectancyMonoclonal antibodiesNonallergic control groupOptimal pharmacological agentsOverall health costsFirst-line therapyAdministration of carboplatinConnective tissue diseaseStandard of careIndividual patient reactionsDesensitized patientsNonallergic groupAllergic groupLine therapyRisk stratificationTissue diseaseHypersensitivity reactionsChronic diseasesOvarian cancerSevere reactionsNonsignificant increasePharmacological agents
2010
Drug Desensitizations in the Management of Allergy and Anaphylaxis to Chemotherapeutic Agents and Monoclonal Antibodies
Banerji A, Brennan P, Hesterberg P, Oren E, Hsu F. Drug Desensitizations in the Management of Allergy and Anaphylaxis to Chemotherapeutic Agents and Monoclonal Antibodies. 2010, 297-311. DOI: 10.1007/978-1-60327-951-2_19.ChaptersDrug hypersensitivity reactionsDrug desensitizationManagement of allergyChemotherapeutic agentsHypersensitivity reactionsMonoclonal antibodiesLife-threatening infectious diseaseDrug-allergic individualsIgE-mediated drug hypersensitivity reactionsMast cell degranulationImmunology specialistsDesensitization protocolRheumatologic diseasesIncremental dosesCautious administrationCell degranulationSensitized individualsInfectious diseasesSmall molecule therapeuticsAllergyDesensitizationNumerous cancersAntibodiesDiseaseDrugs
2009
Hypersensitivity reactions to mAbs: 105 desensitizations in 23 patients, from evaluation to treatment
Brennan PJ, Bouza T, Hsu FI, Sloane DE, Castells MC. Hypersensitivity reactions to mAbs: 105 desensitizations in 23 patients, from evaluation to treatment. Journal Of Allergy And Clinical Immunology 2009, 124: 1259-1266. PMID: 19910036, DOI: 10.1016/j.jaci.2009.09.009.Peer-Reviewed Original ResearchChapter 19 Biology of Mast Cells and their Mediators
Hsu F, Boyce J. Chapter 19 Biology of Mast Cells and their Mediators. 2009, 311-328. DOI: 10.1016/b978-0-323-05659-5.00019-x.Chapters
2008
Hypersensitivity reactions to chemotherapy: Outcomes and safety of rapid desensitization in 413 cases
Castells MC, Tennant NM, Sloane DE, Hsu FI, Barrett NA, Hong DI, Laidlaw TM, Legere HJ, Nallamshetty SN, Palis RI, Rao JJ, Berlin ST, Campos SM, Matulonis UA. Hypersensitivity reactions to chemotherapy: Outcomes and safety of rapid desensitization in 413 cases. Journal Of Allergy And Clinical Immunology 2008, 122: 574-580. PMID: 18502492, DOI: 10.1016/j.jaci.2008.02.044.Peer-Reviewed Original ResearchConceptsHypersensitivity reactionsRapid desensitizationLife-threatening hypersensitivity reactionMedical intensive care unitChemotherapeutic drugsRapid desensitization protocolRapid drug desensitizationIntensive care unitStandard of careFirst desensitizationTemporary tolerizationDesensitization protocolDrug desensitizationCare unitDrug allergensPreferred agentOutpatient settingIntraperitoneal routeSubsequent infusionLiposomal doxorubicinAllergic reactionsAlternative drugsInitial desensitizationTarget doseTreatment recordsAnaphylactic and anaphylactoid reactions to chemotherapy: outcomes and safety of rapid intravenous and intraperitoneal desensitizations in 413 cases
Castells M, Tennant N, Sloane D, Hsu F, Berlin S, Campos S, Matulonis U. Anaphylactic and anaphylactoid reactions to chemotherapy: outcomes and safety of rapid intravenous and intraperitoneal desensitizations in 413 cases. Journal Of Clinical Oncology 2008, 26: 5526-5526. DOI: 10.1200/jco.2008.26.15_suppl.5526.Peer-Reviewed Original Research